PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 31395877-2 2019 Fe-S clusters are biosynthesized on the scaffold protein ISCU, with cysteine desulfurase NFS1 providing sulfur as persulfide and ferredoxin FDX2 supplying electrons, in a process stimulated by FXN but not clearly understood. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 57-61 32131593-3 2020 In the ISC pathway, the pyridoxal 5"-phosphate-dependent cysteine desulfurase enzyme IscS provides sulfur to the scaffold protein IscU, which templates the Fe-S cluster assembly. Iron 156-160 iron-sulfur cluster assembly enzyme Homo sapiens 130-134 31344531-6 2019 By 2D NMR, we confirmed that iron can be bound in both oxidation states, a controversial issue, and, in addition, we were able to point out a transient interaction of frataxin with a N-terminal 6his-tagged variant of ISCU, the scaffold protein of the FeS clusters assembly machinery. Iron 29-33 iron-sulfur cluster assembly enzyme Homo sapiens 217-221 31395877-3 2019 Here, we report the breakdown of this process, made possible by removing a zinc ion in ISCU that hinders iron insertion and promotes non-physiological Fe-S cluster synthesis from free sulfide in vitro. Iron 105-109 iron-sulfur cluster assembly enzyme Homo sapiens 87-91 31395877-3 2019 Here, we report the breakdown of this process, made possible by removing a zinc ion in ISCU that hinders iron insertion and promotes non-physiological Fe-S cluster synthesis from free sulfide in vitro. Iron 151-153 iron-sulfur cluster assembly enzyme Homo sapiens 87-91 31395877-4 2019 By binding zinc-free ISCU, iron drives persulfide uptake from NFS1 and allows persulfide reduction into sulfide by FDX2, thereby coordinating sulfide production with its availability to generate Fe-S clusters. Iron 27-31 iron-sulfur cluster assembly enzyme Homo sapiens 21-25 31395877-4 2019 By binding zinc-free ISCU, iron drives persulfide uptake from NFS1 and allows persulfide reduction into sulfide by FDX2, thereby coordinating sulfide production with its availability to generate Fe-S clusters. Iron 195-197 iron-sulfur cluster assembly enzyme Homo sapiens 21-25 30586625-8 2019 IRE-IRP interaction was affected due to conversion of IRP1 to cytosolic aconitase that was influenced by increased iron-sulfur scaffold protein iron-sulfur cluster assembly enzyme (ISCU) level. Iron 115-119 iron-sulfur cluster assembly enzyme Homo sapiens 181-185 31101807-1 2019 The core machinery for de novo biosynthesis of iron-sulfur clusters (ISC), located in the mitochondria matrix, is a five-protein complex containing the cysteine desulfurase NFS1 that is activated by frataxin (FXN), scaffold protein ISCU, accessory protein ISD11, and acyl-carrier protein ACP. Iron 47-51 iron-sulfur cluster assembly enzyme Homo sapiens 232-236 30586625-8 2019 IRE-IRP interaction was affected due to conversion of IRP1 to cytosolic aconitase that was influenced by increased iron-sulfur scaffold protein iron-sulfur cluster assembly enzyme (ISCU) level. Iron 144-148 iron-sulfur cluster assembly enzyme Homo sapiens 181-185 30557609-6 2019 Over expression of ISCU (Iron-sulfur cluster assembly enzyme, a mitochondrial protein) significantly attenuated DHA induced ferroptosis by regulating iron metabolism, rescuing the mitochondrial function and increasing the level of GSH. Iron 25-29 iron-sulfur cluster assembly enzyme Homo sapiens 19-23 30557609-6 2019 Over expression of ISCU (Iron-sulfur cluster assembly enzyme, a mitochondrial protein) significantly attenuated DHA induced ferroptosis by regulating iron metabolism, rescuing the mitochondrial function and increasing the level of GSH. Iron 150-154 iron-sulfur cluster assembly enzyme Homo sapiens 19-23 30209894-1 2018 BACKGROUND: Hereditary myopathy with lactic acidosis (HML) is an autosomal recessive disease caused by an intron mutation in the iron-sulfur cluster assembly (ISCU) gene. Iron 129-133 iron-sulfur cluster assembly enzyme Homo sapiens 159-163 30031876-1 2018 Human de novo iron-sulfur (Fe-S) assembly complex consists of cysteine desulfurase NFS1, accessory protein ISD11, acyl carrier protein ACP, scaffold protein ISCU, and allosteric activator frataxin (FXN). Iron 27-31 iron-sulfur cluster assembly enzyme Homo sapiens 157-161 30031876-2 2018 FXN binds the NFS1-ISD11-ACP-ISCU complex (SDAU), to activate the desulfurase activity and Fe-S cluster biosynthesis. Iron 91-95 iron-sulfur cluster assembly enzyme Homo sapiens 29-33 30031876-4 2018 Recent studies also reported a substitution at position Met141 on the yeast ISCU orthologue Isu, to Ile, Leu, Val, or Cys, could bypass the requirement of FXN for Fe-S cluster biosynthesis and cell viability. Iron 163-167 iron-sulfur cluster assembly enzyme Homo sapiens 76-80 29782976-2 2018 The biogenesis of Fe-S clusters is orchestrated by ISC system; the sulfur donor IscS and scaffold protein IscU being its core components. Iron 18-22 iron-sulfur cluster assembly enzyme Homo sapiens 106-110 29689452-3 2018 In eukaryotes a [2Fe-2S] cluster is first assembled in the mitochondria on the iron-sulfur cluster scaffold protein ISCU in tandem with iron, sulfide, and electron donors. Iron 79-83 iron-sulfur cluster assembly enzyme Homo sapiens 116-120 29983374-1 2018 Cysteine desulfurase plays a central role in mitochondrial iron-sulfur cluster biogenesis by generating sulfur through the conversion of L-cysteine to L-alanine and by serving as the platform for assembling other components of the biosynthetic machinery, including ISCU, frataxin, and ferredoxin. Iron 59-63 iron-sulfur cluster assembly enzyme Homo sapiens 265-269 29689452-3 2018 In eukaryotes a [2Fe-2S] cluster is first assembled in the mitochondria on the iron-sulfur cluster scaffold protein ISCU in tandem with iron, sulfide, and electron donors. Iron 136-140 iron-sulfur cluster assembly enzyme Homo sapiens 116-120 29802141-3 2018 report a novel cellular system that creates an acute Fe-S cluster deficiency, using mutants of ISCU, the main scaffold protein for Fe-S cluster assembly. Iron 53-57 iron-sulfur cluster assembly enzyme Homo sapiens 95-99 29576242-0 2018 Interactions of iron-bound frataxin with ISCU and ferredoxin on the cysteine desulfurase complex leading to Fe-S cluster assembly. Iron 16-20 iron-sulfur cluster assembly enzyme Homo sapiens 41-45 29576242-0 2018 Interactions of iron-bound frataxin with ISCU and ferredoxin on the cysteine desulfurase complex leading to Fe-S cluster assembly. Iron 108-112 iron-sulfur cluster assembly enzyme Homo sapiens 41-45 28271877-4 2017 The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, thereby forming a quaternary complex for FeS cluster formation. Iron 141-144 iron-sulfur cluster assembly enzyme Homo sapiens 63-67 29596470-10 2018 Interestingly, we show that hypoxia promotes the downregulation of several proteins (ISCU, NFS1, FXN) involved in the early steps of mitochondrial Fe-S cluster biogenesis. Iron 147-151 iron-sulfur cluster assembly enzyme Homo sapiens 85-89 29572489-4 2018 Here, we report that Irp1 or Irp2-null mutation also causes downregulation of frataxin and IscU, two of the core components in the iron-sulfur cluster biogenesis machinery. Iron 131-135 iron-sulfur cluster assembly enzyme Homo sapiens 91-95 29572489-6 2018 Overexpression of human ISCU and frataxin in Irp1 or Irp2-null cells was able to rescue the defects in iron-sulfur cluster biogenesis and mitochondrial quality. Iron 103-107 iron-sulfur cluster assembly enzyme Homo sapiens 24-28 29210568-1 2018 IscU, the central scaffold protein in the bacterial ISC iron-sulfur (Fe-S) cluster biosynthesis system, has long been recognized to bind a Zn2+ ion at its active site. Iron 69-73 iron-sulfur cluster assembly enzyme Homo sapiens 0-4 29210568-2 2018 While initially regarded as an artifact, Zn2+ binding has been shown to induce stabilization of the IscU structure that may mimic a state biologically relevant to IscU"s role in Fe-S cluster biosynthesis. Iron 178-182 iron-sulfur cluster assembly enzyme Homo sapiens 100-104 29210568-2 2018 While initially regarded as an artifact, Zn2+ binding has been shown to induce stabilization of the IscU structure that may mimic a state biologically relevant to IscU"s role in Fe-S cluster biosynthesis. Iron 178-182 iron-sulfur cluster assembly enzyme Homo sapiens 163-167 29210568-7 2018 Monitoring and comparing the conformational behavior and stabilization afforded by different transition metal ions upon IscU and SufU revealed similarities between the two proteins and suggest that metal-dependent conformational transitions may be characteristic of U-type proteins involved in Fe-S cluster biosynthesis. Iron 294-298 iron-sulfur cluster assembly enzyme Homo sapiens 120-124 29406711-1 2018 Whereas iron-sulfur (Fe-S) cluster assembly on the wild-type scaffold protein ISCU, as catalyzed by the human cysteine desulfurase complex (NIA)2, exhibits a requirement for frataxin (FXN), in yeast, ISCU variant M108I has been shown to bypass the FXN requirement. Iron 21-25 iron-sulfur cluster assembly enzyme Homo sapiens 78-82 29406711-1 2018 Whereas iron-sulfur (Fe-S) cluster assembly on the wild-type scaffold protein ISCU, as catalyzed by the human cysteine desulfurase complex (NIA)2, exhibits a requirement for frataxin (FXN), in yeast, ISCU variant M108I has been shown to bypass the FXN requirement. Iron 21-25 iron-sulfur cluster assembly enzyme Homo sapiens 200-204 29406711-4 2018 We have compared the properties of ISCU, ISCU(M108I), and ISCU(D39V), with and without FXN, in both the cysteine desulfurase step of Fe-S cluster assembly and the overall Fe-S cluster assembly reaction catalyzed by (NIA)2. Iron 133-137 iron-sulfur cluster assembly enzyme Homo sapiens 35-39 28664160-6 2017 Its application to the iron-sulfur cluster scaffold protein IscU, which has been suggested to function both as an ordered and a disordered form, allows us to distinguish evolutionary traces of the structured species, suggesting that, if present in the cell, the disordered form has not left evolutionary imprinting. Iron 23-27 iron-sulfur cluster assembly enzyme Homo sapiens 60-64 28380382-3 2017 Here we report that a transient subcomplex involved in CIII assembly, composed of LYRM7 bound to UQCRFS1, interacts with components of an Fe-S transfer complex, consisting of HSC20, its cognate chaperone HSPA9, and the holo-scaffold ISCU. Iron 138-140 iron-sulfur cluster assembly enzyme Homo sapiens 233-237 28001042-9 2017 In an in vitro reaction, the reduced form of each ferredoxin was found to support Fe-S cluster assembly on ISCU; the rate of cluster assembly was faster with FDX2 than with FDX1. Iron 82-86 iron-sulfur cluster assembly enzyme Homo sapiens 107-111 28882199-3 2017 Early studies in yeast and human mitochondria indicated that Fe-S cluster assembly in eukaryotes is centered around highly conserved Fe-S proteins (human ISCU) that serve as scaffolds upon which new Fe-S clusters are assembled from (i) elemental sulfur, provided by a pyridoxal phosphate-dependent cysteine desulfurase (human NFS1) and its stabilizing-binding partner (human ISD11), and (ii) elemental iron, provided by an iron-binding protein of the frataxin family (human FXN). Iron 61-65 iron-sulfur cluster assembly enzyme Homo sapiens 154-158 28882199-3 2017 Early studies in yeast and human mitochondria indicated that Fe-S cluster assembly in eukaryotes is centered around highly conserved Fe-S proteins (human ISCU) that serve as scaffolds upon which new Fe-S clusters are assembled from (i) elemental sulfur, provided by a pyridoxal phosphate-dependent cysteine desulfurase (human NFS1) and its stabilizing-binding partner (human ISD11), and (ii) elemental iron, provided by an iron-binding protein of the frataxin family (human FXN). Iron 61-63 iron-sulfur cluster assembly enzyme Homo sapiens 154-158 28882199-3 2017 Early studies in yeast and human mitochondria indicated that Fe-S cluster assembly in eukaryotes is centered around highly conserved Fe-S proteins (human ISCU) that serve as scaffolds upon which new Fe-S clusters are assembled from (i) elemental sulfur, provided by a pyridoxal phosphate-dependent cysteine desulfurase (human NFS1) and its stabilizing-binding partner (human ISD11), and (ii) elemental iron, provided by an iron-binding protein of the frataxin family (human FXN). Iron 402-406 iron-sulfur cluster assembly enzyme Homo sapiens 154-158 28882199-3 2017 Early studies in yeast and human mitochondria indicated that Fe-S cluster assembly in eukaryotes is centered around highly conserved Fe-S proteins (human ISCU) that serve as scaffolds upon which new Fe-S clusters are assembled from (i) elemental sulfur, provided by a pyridoxal phosphate-dependent cysteine desulfurase (human NFS1) and its stabilizing-binding partner (human ISD11), and (ii) elemental iron, provided by an iron-binding protein of the frataxin family (human FXN). Iron 423-427 iron-sulfur cluster assembly enzyme Homo sapiens 154-158 27783661-1 2016 Hereditary myopathy with lactic acidosis (HML) is an autosomal recessive disease caused by an intronic one-base mutation in the iron-sulfur cluster assembly (ISCU) gene, resulting in aberrant splicing. Iron 128-132 iron-sulfur cluster assembly enzyme Homo sapiens 158-162 27653419-6 2016 A bacterial Fe-S assembly complex, composed of the cysteine desulfurase IscS and scaffold protein IscU, was used to generate [2Fe-2S] clusters for transfer to mixtures of putative intermediate carrier and acceptor proteins. Iron 12-16 iron-sulfur cluster assembly enzyme Homo sapiens 98-102 27590019-1 2016 Glutathione-coordinated [2Fe-2S] complex is a non-protein-bound [2Fe-2S] cluster that is capable of reconstituting the human iron-sulfur cluster scaffold protein IscU. Iron 125-129 iron-sulfur cluster assembly enzyme Homo sapiens 162-166 27714045-2 2016 In most organisms, Fe-S clusters are initially assembled on a scaffold protein, ISCU, and subsequently transferred to target proteins or to intermediate carriers by a dedicated chaperone/co-chaperone system. Iron 19-23 iron-sulfur cluster assembly enzyme Homo sapiens 80-84 27714045-3 2016 The delivery of assembled Fe-S clusters to recipient proteins is a crucial step in the biogenesis of Fe-S proteins, and, in mammals, it relies on the activity of a multiprotein transfer complex that contains the chaperone HSPA9, the co-chaperone HSC20 and the scaffold ISCU. Iron 26-30 iron-sulfur cluster assembly enzyme Homo sapiens 269-273 27714045-3 2016 The delivery of assembled Fe-S clusters to recipient proteins is a crucial step in the biogenesis of Fe-S proteins, and, in mammals, it relies on the activity of a multiprotein transfer complex that contains the chaperone HSPA9, the co-chaperone HSC20 and the scaffold ISCU. Iron 101-105 iron-sulfur cluster assembly enzyme Homo sapiens 269-273 27783661-2 2016 The incorrectly spliced transcripts contain a 100 or 86 bp intron sequence encoding a non-functional ISCU protein, which leads to defects in several Fe-S containing proteins in the respiratory chain and the TCA cycle. Iron 149-153 iron-sulfur cluster assembly enzyme Homo sapiens 101-105 27519411-9 2016 The organization of each center recapitulates a previously proposed conserved mechanism for sulfur donation from NFS1 to ISCU and reveals, for the first time, a path for iron donation from FXN42-210 to ISCU. Iron 170-174 iron-sulfur cluster assembly enzyme Homo sapiens 202-206 26342079-2 2015 In mammalian mitochondria, the initial step of the Fe-S cluster assembly process is assisted by the NFS1-ISD11 complex, which delivers sulfur to scaffold protein ISCU during Fe-S cluster synthesis. Iron 51-55 iron-sulfur cluster assembly enzyme Homo sapiens 162-166 26749241-3 2016 SDHAF1 transiently binds to aromatic peptides of SDHB through an arginine-rich region in its C terminus and specifically engages a Fe-S donor complex, consisting of the scaffold, holo-ISCU, and the co-chaperone-chaperone pair, HSC20-HSPA9, through an LYR motif near its N-terminal domain. Iron 131-135 iron-sulfur cluster assembly enzyme Homo sapiens 184-188 26342079-2 2015 In mammalian mitochondria, the initial step of the Fe-S cluster assembly process is assisted by the NFS1-ISD11 complex, which delivers sulfur to scaffold protein ISCU during Fe-S cluster synthesis. Iron 174-178 iron-sulfur cluster assembly enzyme Homo sapiens 162-166 24111776-5 2014 Several miR-210 target genes, including iron-sulfur (Fe-S) cluster scaffold protein (ISCU) and glycerol-3-phosphate dehydrogenase 1-like (GPD1L), have been correlated with prognosis in an inverse fashion to miR-210, suggesting that their down- regulation by miR-210 occurs in vivo and contributes to tumor growth. Iron 53-55 iron-sulfur cluster assembly enzyme Homo sapiens 85-89 26032732-0 2015 Interaction of frataxin, an iron binding protein, with IscU of Fe-S clusters biogenesis pathway and its upregulation in AmpB resistant Leishmania donovani. Iron 28-32 iron-sulfur cluster assembly enzyme Homo sapiens 55-59 26032732-0 2015 Interaction of frataxin, an iron binding protein, with IscU of Fe-S clusters biogenesis pathway and its upregulation in AmpB resistant Leishmania donovani. Iron 63-67 iron-sulfur cluster assembly enzyme Homo sapiens 55-59 25447544-0 2015 Anatomy of an iron-sulfur cluster scaffold protein: Understanding the determinants of [2Fe-2S] cluster stability on IscU. Iron 14-18 iron-sulfur cluster assembly enzyme Homo sapiens 116-120 25597503-2 2015 In mammals, the primary steps of Fe-S cluster assembly are performed by the NFS1-ISD11-ISCU complex via the formation of a persulfide intermediate on NFS1. Iron 33-37 iron-sulfur cluster assembly enzyme Homo sapiens 87-91 24976419-10 2014 Sustained ISCU protein levels enhanced by mTORC1 can inhibit iron-responsive element and iron-regulatory protein binding activities. Iron 61-65 iron-sulfur cluster assembly enzyme Homo sapiens 10-14 24917298-2 2014 Here, we have combined experimental and computational methods to study the stability of IscU, a bacterial scaffold protein highly conserved in most organisms and an essential component of the iron-sulfur cluster biogenesis pathway. Iron 192-196 iron-sulfur cluster assembly enzyme Homo sapiens 88-92 24606901-1 2014 Iron sulfur (Fe-S) clusters, preassembled on the ISCU scaffold, are transferred to target proteins or to intermediate scaffolds by a dedicated chaperone-cochaperone system. Iron 13-17 iron-sulfur cluster assembly enzyme Homo sapiens 49-53 25044349-0 2014 The scaffold protein IscU retains a structured conformation in the Fe-S cluster assembly complex. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 21-25 25044349-1 2014 IscU and IscS are two essential proteins in the machine responsible for the biogenesis of iron-sulfur clusters, prosthetic groups that are involved in several essential functions. Iron 90-94 iron-sulfur cluster assembly enzyme Homo sapiens 0-4 24281368-6 2014 Fdx2 is the second component of the Fe-S cluster biogenesis machinery, the first being IscU that is associated with isolated mitochondrial myopathy. Iron 36-40 iron-sulfur cluster assembly enzyme Homo sapiens 87-91 24573684-2 2014 Initially, the Fe-S clusters are assembled on a conserved scaffold protein, iron-sulfur cluster scaffold protein (ISCU), in coordination with iron and sulfur donor proteins in human mitochondria. Iron 15-19 iron-sulfur cluster assembly enzyme Homo sapiens 114-118 24573684-2 2014 Initially, the Fe-S clusters are assembled on a conserved scaffold protein, iron-sulfur cluster scaffold protein (ISCU), in coordination with iron and sulfur donor proteins in human mitochondria. Iron 76-80 iron-sulfur cluster assembly enzyme Homo sapiens 114-118 24606901-4 2014 In succinate dehydrogenase B, two LYR motifs engage the ISCU-HSC20-HSPA9 complex to aid incorporation of three Fe-S clusters within the final structure of complex II. Iron 111-115 iron-sulfur cluster assembly enzyme Homo sapiens 56-60 24498631-2 2014 Mutations in Fe-S proteins often disrupt Fe-S cluster assembly leading to a spectrum of severe disorders such as Friedreich"s ataxia or iron-sulfur cluster assembly enzyme (ISCU) myopathy. Iron 13-15 iron-sulfur cluster assembly enzyme Homo sapiens 173-177 24498631-2 2014 Mutations in Fe-S proteins often disrupt Fe-S cluster assembly leading to a spectrum of severe disorders such as Friedreich"s ataxia or iron-sulfur cluster assembly enzyme (ISCU) myopathy. Iron 13-17 iron-sulfur cluster assembly enzyme Homo sapiens 173-177 24498631-2 2014 Mutations in Fe-S proteins often disrupt Fe-S cluster assembly leading to a spectrum of severe disorders such as Friedreich"s ataxia or iron-sulfur cluster assembly enzyme (ISCU) myopathy. Iron 136-140 iron-sulfur cluster assembly enzyme Homo sapiens 173-177 23137639-0 2013 Iron-sulfur cluster scaffold (ISCU) gene is duplicated in salmonid fish and tissue and temperature dependent expressed in rainbow trout. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 30-34 23909240-4 2013 Further, we demonstrate that His86 in this N-terminal region is required for high affinity iron coordination and iron assembly of Fe-S clusters by ISCU as part of the Fe-S cluster biosynthetic complex. Iron 91-95 iron-sulfur cluster assembly enzyme Homo sapiens 147-151 23909240-4 2013 Further, we demonstrate that His86 in this N-terminal region is required for high affinity iron coordination and iron assembly of Fe-S clusters by ISCU as part of the Fe-S cluster biosynthetic complex. Iron 113-117 iron-sulfur cluster assembly enzyme Homo sapiens 147-151 23909240-4 2013 Further, we demonstrate that His86 in this N-terminal region is required for high affinity iron coordination and iron assembly of Fe-S clusters by ISCU as part of the Fe-S cluster biosynthetic complex. Iron 130-134 iron-sulfur cluster assembly enzyme Homo sapiens 147-151 23909240-4 2013 Further, we demonstrate that His86 in this N-terminal region is required for high affinity iron coordination and iron assembly of Fe-S clusters by ISCU as part of the Fe-S cluster biosynthetic complex. Iron 167-171 iron-sulfur cluster assembly enzyme Homo sapiens 147-151 23796308-3 2013 Recent reports have highlighted the existence of structural flexibility in the ISU/IscU-type scaffold proteins that mediate Fe-S cluster assembly, which is also likely to serve an important role in the pathway to Fe-S cluster maturation. Iron 124-128 iron-sulfur cluster assembly enzyme Homo sapiens 83-87 23796308-3 2013 Recent reports have highlighted the existence of structural flexibility in the ISU/IscU-type scaffold proteins that mediate Fe-S cluster assembly, which is also likely to serve an important role in the pathway to Fe-S cluster maturation. Iron 213-217 iron-sulfur cluster assembly enzyme Homo sapiens 83-87 23846788-6 2013 To date, miRNA have been demonstrated to post-transcriptionally regulate the expression of genes associated with iron acquisition (transferrin receptor and divalent metal transporter), iron export (ferroportin), iron storage (ferritin), iron utilization (ISCU), and coordination of systemic iron homeostasis (HFE and hemojevelin). Iron 113-117 iron-sulfur cluster assembly enzyme Homo sapiens 255-259 23846788-6 2013 To date, miRNA have been demonstrated to post-transcriptionally regulate the expression of genes associated with iron acquisition (transferrin receptor and divalent metal transporter), iron export (ferroportin), iron storage (ferritin), iron utilization (ISCU), and coordination of systemic iron homeostasis (HFE and hemojevelin). Iron 185-189 iron-sulfur cluster assembly enzyme Homo sapiens 255-259 23846788-6 2013 To date, miRNA have been demonstrated to post-transcriptionally regulate the expression of genes associated with iron acquisition (transferrin receptor and divalent metal transporter), iron export (ferroportin), iron storage (ferritin), iron utilization (ISCU), and coordination of systemic iron homeostasis (HFE and hemojevelin). Iron 185-189 iron-sulfur cluster assembly enzyme Homo sapiens 255-259 23846788-6 2013 To date, miRNA have been demonstrated to post-transcriptionally regulate the expression of genes associated with iron acquisition (transferrin receptor and divalent metal transporter), iron export (ferroportin), iron storage (ferritin), iron utilization (ISCU), and coordination of systemic iron homeostasis (HFE and hemojevelin). Iron 185-189 iron-sulfur cluster assembly enzyme Homo sapiens 255-259 23508953-0 2013 Mammalian target of rapamycin complex 1 (mTORC1)-mediated phosphorylation stabilizes ISCU protein: implications for iron metabolism. Iron 116-120 iron-sulfur cluster assembly enzyme Homo sapiens 85-89 23508953-1 2013 The scaffold protein ISCU facilitates the assembly of iron-sulfur clusters (ISCs), which are essential cofactors for many vital metabolic processes. Iron 54-58 iron-sulfur cluster assembly enzyme Homo sapiens 21-25 23508953-6 2013 Sustained ISCU protein levels enhanced by mTORC1 sensitized TSC2-null cells to iron deprivation due to constitutive ISC biogenesis-triggered iron demand, which outstrips supply. Iron 79-83 iron-sulfur cluster assembly enzyme Homo sapiens 10-14 23508953-6 2013 Sustained ISCU protein levels enhanced by mTORC1 sensitized TSC2-null cells to iron deprivation due to constitutive ISC biogenesis-triggered iron demand, which outstrips supply. Iron 141-145 iron-sulfur cluster assembly enzyme Homo sapiens 10-14 23586717-6 2013 Further studies indicate that the iron-loaded IscA could act as an iron donor for the assembly of iron-sulfur clusters in the scaffold protein IscU in vitro. Iron 34-38 iron-sulfur cluster assembly enzyme Homo sapiens 143-147 23586717-6 2013 Further studies indicate that the iron-loaded IscA could act as an iron donor for the assembly of iron-sulfur clusters in the scaffold protein IscU in vitro. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 143-147 23586717-6 2013 Further studies indicate that the iron-loaded IscA could act as an iron donor for the assembly of iron-sulfur clusters in the scaffold protein IscU in vitro. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 143-147 23265191-4 2013 Here, by comparing the biochemical and spectroscopic properties of quaternary (ISCU/NFS1/ISD11/FXN) and ternary (ISCU/NFS1/ISD11) complexes, we show that FXN stabilizes the quaternary complex and controls iron entry to the complex through activation of cysteine desulfurization. Iron 205-209 iron-sulfur cluster assembly enzyme Homo sapiens 79-83 23265191-4 2013 Here, by comparing the biochemical and spectroscopic properties of quaternary (ISCU/NFS1/ISD11/FXN) and ternary (ISCU/NFS1/ISD11) complexes, we show that FXN stabilizes the quaternary complex and controls iron entry to the complex through activation of cysteine desulfurization. Iron 205-209 iron-sulfur cluster assembly enzyme Homo sapiens 113-117 23137639-1 2013 The iron-sulfur cluster protein ISCU is a scaffold protein tasked with the building and mediation of iron-sulfur [Fe-S]-clusters. Iron 4-8 iron-sulfur cluster assembly enzyme Homo sapiens 32-36 23137639-1 2013 The iron-sulfur cluster protein ISCU is a scaffold protein tasked with the building and mediation of iron-sulfur [Fe-S]-clusters. Iron 101-105 iron-sulfur cluster assembly enzyme Homo sapiens 32-36 23137639-1 2013 The iron-sulfur cluster protein ISCU is a scaffold protein tasked with the building and mediation of iron-sulfur [Fe-S]-clusters. Iron 114-118 iron-sulfur cluster assembly enzyme Homo sapiens 32-36 22896707-6 2012 Furthermore we found two essential molecules for iron homeostasis, iron-sulfur cluster scaffold protein (ISCU) and transferrin receptor 1 (TfR), are a direct target of miR-210. Iron 49-53 iron-sulfur cluster assembly enzyme Homo sapiens 105-109 23035118-4 2012 SIGNIFICANCE: Oxidative stress negatively influences the mammalian Fe-S cluster assembly machinery by destabilization of ISCU. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 121-125 23035118-5 2012 Iron-sulfur (Fe-S) cluster cofactors are formed on the scaffold protein ISCU. Iron 13-17 iron-sulfur cluster assembly enzyme Homo sapiens 72-76 22155317-2 2012 The disease is recessively inherited and caused by a deep intronic, single base transition in the iron-sulfur cluster scaffold, ISCU gene that causes retention of a pseudoexon and introduction of a premature termination codon. Iron 98-102 iron-sulfur cluster assembly enzyme Homo sapiens 128-132 22125086-1 2012 Hereditary myopathy with lactic acidosis (HML) is caused by an intron mutation in the iron-sulfur cluster assembly gene ISCU, which leads to the activation of cryptic splice sites and the retention of part of intron 4. Iron 86-90 iron-sulfur cluster assembly enzyme Homo sapiens 120-124 22155317-3 2012 IscU protein deficiency causes secondary defects in several iron-sulfur dependant proteins, including enzymes involved in aerobic energy metabolism. Iron 60-64 iron-sulfur cluster assembly enzyme Homo sapiens 0-4 21945528-3 2011 We were able to experimentally confirm a role of Fdx1 in the iron-sulfur cluster biosynthesis by in vitro reduction studies with cluster-loaded So ce56 IscU and by transfer studies of the cluster from the latter protein to apo-aconitase A. Iron 61-65 iron-sulfur cluster assembly enzyme Homo sapiens 152-156 21987576-7 2011 Isa1 and Isa2 proteins are shown to bind iron in vivo, yet the Isa1-Isa2-bound iron was not needed as a donor for de novo assembly of the [2Fe-2S] cluster on the general Fe/S scaffold proteins Isu1-Isu2. Iron 79-83 iron-sulfur cluster assembly enzyme Homo sapiens 198-202 20957368-4 2011 The CyaY protein can bind ferric iron and serve as an iron donor for the biogenesis of iron-sulfur clusters on the scaffold protein IscU in the presence of IscS and L-cysteine in vitro. Iron 33-37 iron-sulfur cluster assembly enzyme Homo sapiens 132-136 21801864-8 2011 RNA interference knockdown of ISCU expression in Swan 71, but not in BeWo, cells resulted in autophagosomal and siderosomal iron accumulation and a fourfold decrease of Matrigel invasion (P = 0.004). Iron 124-128 iron-sulfur cluster assembly enzyme Homo sapiens 30-34 21801864-12 2011 The findings herein suggest that ISCU down-regulation by miR-210 perturbing trophoblast iron metabolism is associated with defective placentation. Iron 88-92 iron-sulfur cluster assembly enzyme Homo sapiens 33-37 21671584-10 2011 Together, these results suggest that Fxn facilitates sulfur transfer from Nfs1 to Isu2 and that these in vitro assays are sensitive and appropriate for deciphering functional defects and mechanistic details for human Fe-S cluster biosynthesis. Iron 217-221 iron-sulfur cluster assembly enzyme Homo sapiens 82-86 21499539-5 2011 This review summarizes some key structural and functional properties of three central proteins dedicated to the Fe-S cluster assembly process: namely, the sulfide donor (cysteine desulfurase); iron donor (frataxin), and the iron-sulfur cluster scaffold protein (IscU/ISU). Iron 112-116 iron-sulfur cluster assembly enzyme Homo sapiens 262-266 21499539-5 2011 This review summarizes some key structural and functional properties of three central proteins dedicated to the Fe-S cluster assembly process: namely, the sulfide donor (cysteine desulfurase); iron donor (frataxin), and the iron-sulfur cluster scaffold protein (IscU/ISU). Iron 193-197 iron-sulfur cluster assembly enzyme Homo sapiens 262-266 20957368-4 2011 The CyaY protein can bind ferric iron and serve as an iron donor for the biogenesis of iron-sulfur clusters on the scaffold protein IscU in the presence of IscS and L-cysteine in vitro. Iron 54-58 iron-sulfur cluster assembly enzyme Homo sapiens 132-136 20981023-2 2010 Independent reports have linked frataxin to iron-sulphur cluster assembly through interactions with the two central components of this machinery: desulphurase Nfs1/IscS and the scaffold protein Isu/IscU. Iron 44-48 iron-sulfur cluster assembly enzyme Homo sapiens 198-202 21298097-4 2011 Through immunoprecipitation experiments, we show that the main endogenous interactors of a recombinant mature human frataxin are ISCU, NFS1 and ISD11, the components of the core Fe-S assembly complex. Iron 178-182 iron-sulfur cluster assembly enzyme Homo sapiens 129-133 20873749-3 2010 Here we provide in vitro evidence that human frataxin binds to a Nfs1, Isd11, and Isu2 complex to generate the four-component core machinery for Fe-S cluster biosynthesis. Iron 145-149 iron-sulfur cluster assembly enzyme Homo sapiens 82-86 20206689-1 2010 Hereditary myopathy with lactic acidosis, or myopathy with exercise intolerance, Swedish type (OMIM #255125) is caused by mutations in the iron-sulfur cluster scaffold (ISCU) gene. Iron 139-143 iron-sulfur cluster assembly enzyme Homo sapiens 169-173 20481466-5 2010 Fe-S cluster biogenesis takes place essentially in every tissue of humans, and products of human disease genes, including frataxin, GLRX5, ISCU, and ABCB7, have important roles in the process. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 139-143 20206689-5 2010 Consistent with the recognised role of ISCU, we found abnormal activities of respiratory chain complexes containing iron-sulfur clusters in patient muscle. Iron 116-120 iron-sulfur cluster assembly enzyme Homo sapiens 39-43 20436681-2 2010 METHODS AND FINDINGS: In human cancer cell lines and tumours, we found that miR-210 targets the mitochondrial iron sulfur scaffold protein ISCU, required for assembly of iron-sulfur clusters, cofactors for key enzymes involved in the Krebs cycle, electron transport, and iron metabolism. Iron 110-114 iron-sulfur cluster assembly enzyme Homo sapiens 139-143 20436681-2 2010 METHODS AND FINDINGS: In human cancer cell lines and tumours, we found that miR-210 targets the mitochondrial iron sulfur scaffold protein ISCU, required for assembly of iron-sulfur clusters, cofactors for key enzymes involved in the Krebs cycle, electron transport, and iron metabolism. Iron 170-174 iron-sulfur cluster assembly enzyme Homo sapiens 139-143 20436681-2 2010 METHODS AND FINDINGS: In human cancer cell lines and tumours, we found that miR-210 targets the mitochondrial iron sulfur scaffold protein ISCU, required for assembly of iron-sulfur clusters, cofactors for key enzymes involved in the Krebs cycle, electron transport, and iron metabolism. Iron 170-174 iron-sulfur cluster assembly enzyme Homo sapiens 139-143 20404999-9 2010 Our data support the role of frataxin as an iron donor for IscU to form the Fe-S clusters. Iron 44-48 iron-sulfur cluster assembly enzyme Homo sapiens 59-63 20404999-9 2010 Our data support the role of frataxin as an iron donor for IscU to form the Fe-S clusters. Iron 76-80 iron-sulfur cluster assembly enzyme Homo sapiens 59-63 19308466-0 2009 Iron-sulfur cluster biosynthesis: characterization of IscU-IscS complex formation and a structural model for sulfide delivery to the [2Fe-2S] assembly site. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 54-58 19846308-1 2009 Iron-sulphur cluster deficiency myopathy is caused by a deep intronic mutation in ISCU resulting in inclusion of a cryptic exon in the mature mRNA. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 82-86 19567699-0 2009 Clinical manifestation and a new ISCU mutation in iron-sulphur cluster deficiency myopathy. Iron 50-54 iron-sulfur cluster assembly enzyme Homo sapiens 33-37 19567699-4 2009 The clinical, histochemical and biochemical phenotype is very homogenous and the patients are homozygous for a deep intronic IVS5 + 382G>C splicing affecting mutation in ISCU, which encodes the differently spliced cytosolic and mitochondrial iron-sulphur cluster assembly protein IscU. Iron 242-246 iron-sulfur cluster assembly enzyme Homo sapiens 170-174 19567699-4 2009 The clinical, histochemical and biochemical phenotype is very homogenous and the patients are homozygous for a deep intronic IVS5 + 382G>C splicing affecting mutation in ISCU, which encodes the differently spliced cytosolic and mitochondrial iron-sulphur cluster assembly protein IscU. Iron 242-246 iron-sulfur cluster assembly enzyme Homo sapiens 280-284 19567699-5 2009 Iron-sulphur cluster containing proteins are essential for iron homeostasis and respiratory chain function, with IscU being among the most conserved proteins in evolution. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 113-117 19567699-5 2009 Iron-sulphur cluster containing proteins are essential for iron homeostasis and respiratory chain function, with IscU being among the most conserved proteins in evolution. Iron 59-63 iron-sulfur cluster assembly enzyme Homo sapiens 113-117 18606475-3 2008 Recently, the spectrum of diseases attributable to abnormal Fe-S cluster biogenesis has extended beyond Friedreich ataxia to include a sideroblastic anemia with deficiency of glutaredoxin 5 and a myopathy associated with a deficiency of a Fe-S cluster assembly scaffold protein, ISCU. Iron 60-64 iron-sulfur cluster assembly enzyme Homo sapiens 279-283 19492851-0 2009 Structure and dynamics of the iron-sulfur cluster assembly scaffold protein IscU and its interaction with the cochaperone HscB. Iron 30-34 iron-sulfur cluster assembly enzyme Homo sapiens 76-80 19492851-1 2009 IscU is a scaffold protein that functions in iron-sulfur cluster assembly and transfer. Iron 45-49 iron-sulfur cluster assembly enzyme Homo sapiens 0-4 19462090-1 2009 His mediates iron delivery to the [2Fe-2S] assembly site of ISU/IscU scaffold proteins, but is not required for the binding of iron or cluster. Iron 13-17 iron-sulfur cluster assembly enzyme Homo sapiens 64-68 18986169-0 2008 Studies on the mechanism of catalysis of iron-sulfur cluster transfer from IscU[2Fe2S] by HscA/HscB chaperones. Iron 41-45 iron-sulfur cluster assembly enzyme Homo sapiens 75-79 18986169-1 2008 The HscA/HscB chaperone/cochaperone system accelerates transfer of iron-sulfur clusters from the FeS-scaffold protein IscU (IscU(2)[2Fe2S], holo-IscU) to acceptor proteins in an ATP-dependent manner. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 118-122 18986169-1 2008 The HscA/HscB chaperone/cochaperone system accelerates transfer of iron-sulfur clusters from the FeS-scaffold protein IscU (IscU(2)[2Fe2S], holo-IscU) to acceptor proteins in an ATP-dependent manner. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 124-138 18986169-1 2008 The HscA/HscB chaperone/cochaperone system accelerates transfer of iron-sulfur clusters from the FeS-scaffold protein IscU (IscU(2)[2Fe2S], holo-IscU) to acceptor proteins in an ATP-dependent manner. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 124-128 18986169-8 2008 These results suggest that acceleration of iron-sulfur cluster transfer involves a structural change in the IscU(2)[2Fe2S] complex during the T --> R transition of HscA accompanying ATP hydrolysis. Iron 43-47 iron-sulfur cluster assembly enzyme Homo sapiens 108-122 18606475-3 2008 Recently, the spectrum of diseases attributable to abnormal Fe-S cluster biogenesis has extended beyond Friedreich ataxia to include a sideroblastic anemia with deficiency of glutaredoxin 5 and a myopathy associated with a deficiency of a Fe-S cluster assembly scaffold protein, ISCU. Iron 239-243 iron-sulfur cluster assembly enzyme Homo sapiens 279-283 18304497-4 2008 A marked reduction of ISCU mRNA and mitochondrial ISCU protein in patient muscle was associated with a decrease in the iron regulatory protein IRP1 and intracellular iron overload in skeletal muscle, consistent with a muscle-specific alteration of iron homeostasis in this disease. Iron 119-123 iron-sulfur cluster assembly enzyme Homo sapiens 22-26 18296749-4 2008 The 1.6-Mb disease-critical region contained one obvious candidate gene-ISCU-specifying a protein involved in iron-sulphur cluster assembly. Iron 110-114 iron-sulfur cluster assembly enzyme Homo sapiens 72-76 18304497-0 2008 Splice mutation in the iron-sulfur cluster scaffold protein ISCU causes myopathy with exercise intolerance. Iron 23-27 iron-sulfur cluster assembly enzyme Homo sapiens 60-64 18304497-2 2008 We identified the gene for the iron-sulfur cluster scaffold protein ISCU as a candidate within a region of shared homozygosity among patients with this disease. Iron 31-35 iron-sulfur cluster assembly enzyme Homo sapiens 68-72 18304497-4 2008 A marked reduction of ISCU mRNA and mitochondrial ISCU protein in patient muscle was associated with a decrease in the iron regulatory protein IRP1 and intracellular iron overload in skeletal muscle, consistent with a muscle-specific alteration of iron homeostasis in this disease. Iron 119-123 iron-sulfur cluster assembly enzyme Homo sapiens 50-54 18304497-4 2008 A marked reduction of ISCU mRNA and mitochondrial ISCU protein in patient muscle was associated with a decrease in the iron regulatory protein IRP1 and intracellular iron overload in skeletal muscle, consistent with a muscle-specific alteration of iron homeostasis in this disease. Iron 166-170 iron-sulfur cluster assembly enzyme Homo sapiens 22-26 18304497-4 2008 A marked reduction of ISCU mRNA and mitochondrial ISCU protein in patient muscle was associated with a decrease in the iron regulatory protein IRP1 and intracellular iron overload in skeletal muscle, consistent with a muscle-specific alteration of iron homeostasis in this disease. Iron 166-170 iron-sulfur cluster assembly enzyme Homo sapiens 50-54 18304497-4 2008 A marked reduction of ISCU mRNA and mitochondrial ISCU protein in patient muscle was associated with a decrease in the iron regulatory protein IRP1 and intracellular iron overload in skeletal muscle, consistent with a muscle-specific alteration of iron homeostasis in this disease. Iron 166-170 iron-sulfur cluster assembly enzyme Homo sapiens 22-26 18304497-4 2008 A marked reduction of ISCU mRNA and mitochondrial ISCU protein in patient muscle was associated with a decrease in the iron regulatory protein IRP1 and intracellular iron overload in skeletal muscle, consistent with a muscle-specific alteration of iron homeostasis in this disease. Iron 166-170 iron-sulfur cluster assembly enzyme Homo sapiens 50-54 18304497-5 2008 ISCU interacts with the Friedreich ataxia gene product frataxin in iron-sulfur cluster biosynthesis. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 0-4 16527810-0 2006 Roles of the mammalian cytosolic cysteine desulfurase, ISCS, and scaffold protein, ISCU, in iron-sulfur cluster assembly. Iron 92-96 iron-sulfur cluster assembly enzyme Homo sapiens 83-87 17331979-4 2007 Another interactor was ISD11, recently identified as a component of the eukaryotic complex Nfs1/ISCU, an essential component of iron-sulfur cluster biogenesis. Iron 128-132 iron-sulfur cluster assembly enzyme Homo sapiens 96-100 17331979-12 2007 These data suggest that frataxin binds the iron-sulfur biogenesis Nfs1/ISCU complex through ISD11, that the interaction is nickel-dependent, and that multiple consequences of frataxin deficiency are duplicated by ISD11 deficiency. Iron 43-47 iron-sulfur cluster assembly enzyme Homo sapiens 71-75 17244611-5 2007 Under the same physiologically relevant conditions, IscA efficiently recruits iron and transfers the iron for the iron-sulfur cluster assembly in a proposed scaffold IscU. Iron 101-105 iron-sulfur cluster assembly enzyme Homo sapiens 166-170 17244611-5 2007 Under the same physiologically relevant conditions, IscA efficiently recruits iron and transfers the iron for the iron-sulfur cluster assembly in a proposed scaffold IscU. Iron 101-105 iron-sulfur cluster assembly enzyme Homo sapiens 166-170 16603772-0 2006 Iron-sulfur cluster biosynthesis: characterization of Escherichia coli CYaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 147-151 16603772-0 2006 Iron-sulfur cluster biosynthesis: characterization of Escherichia coli CYaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU. Iron 82-86 iron-sulfur cluster assembly enzyme Homo sapiens 147-151 16527810-9 2006 When incubated with iron regulatory protein 1, cysteine, and iron, the cytosolic forms of ISCS and ISCU facilitated efficient formation of a [4Fe-4S] cluster on IRP1. Iron 20-24 iron-sulfur cluster assembly enzyme Homo sapiens 99-103 16527810-10 2006 Thus, the cytosolic form of ISCS is a functional cysteine desulfurase that can collaborate with cytosolic ISCU to promote de novo iron-sulfur cluster formation. Iron 130-134 iron-sulfur cluster assembly enzyme Homo sapiens 106-110 16517407-0 2006 Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis. Iron 64-68 iron-sulfur cluster assembly enzyme Homo sapiens 46-50 16517407-0 2006 Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis. Iron 99-103 iron-sulfur cluster assembly enzyme Homo sapiens 46-50 16517407-4 2006 Suppression of human ISCU by RNAi not only inactivated mitochondrial and cytosolic aconitases in a compartment-specific manner but also inappropriately activated the iron regulatory proteins and disrupted intracellular iron homeostasis. Iron 166-170 iron-sulfur cluster assembly enzyme Homo sapiens 21-25 16517407-4 2006 Suppression of human ISCU by RNAi not only inactivated mitochondrial and cytosolic aconitases in a compartment-specific manner but also inappropriately activated the iron regulatory proteins and disrupted intracellular iron homeostasis. Iron 219-223 iron-sulfur cluster assembly enzyme Homo sapiens 21-25 16517407-5 2006 Furthermore, endogenous ISCU levels were suppressed by iron deprivation. Iron 55-59 iron-sulfur cluster assembly enzyme Homo sapiens 24-28 15828873-0 2005 Mobilization of the iron centre in IscA for the iron-sulphur cluster assembly in IscU. Iron 20-24 iron-sulfur cluster assembly enzyme Homo sapiens 81-85 15828873-0 2005 Mobilization of the iron centre in IscA for the iron-sulphur cluster assembly in IscU. Iron 48-52 iron-sulfur cluster assembly enzyme Homo sapiens 81-85 15247288-0 2004 IscA mediates iron delivery for assembly of iron-sulfur clusters in IscU under the limited accessible free iron conditions. Iron 14-18 iron-sulfur cluster assembly enzyme Homo sapiens 68-72 15815978-0 2005 Structural characterization of an iron-sulfur cluster assembly protein IscU in a zinc-bound form. Iron 34-38 iron-sulfur cluster assembly enzyme Homo sapiens 71-75 15766257-2 2005 IscU functions as a scaffold for Fe-S cluster assembly and transfer, and is known to be a substrate protein for molecular chaperones. Iron 33-37 iron-sulfur cluster assembly enzyme Homo sapiens 0-4 15766257-3 2005 Kinetic studies of Fe-S cluster transfer from holo IscU to apo Fd in the presence of chaperone DnaK demonstrate an inhibitory effect on the rate of Fe-S cluster transfer from IscU. Iron 19-23 iron-sulfur cluster assembly enzyme Homo sapiens 51-55 15766257-3 2005 Kinetic studies of Fe-S cluster transfer from holo IscU to apo Fd in the presence of chaperone DnaK demonstrate an inhibitory effect on the rate of Fe-S cluster transfer from IscU. Iron 19-23 iron-sulfur cluster assembly enzyme Homo sapiens 175-179 15766257-3 2005 Kinetic studies of Fe-S cluster transfer from holo IscU to apo Fd in the presence of chaperone DnaK demonstrate an inhibitory effect on the rate of Fe-S cluster transfer from IscU. Iron 148-152 iron-sulfur cluster assembly enzyme Homo sapiens 51-55 15766257-3 2005 Kinetic studies of Fe-S cluster transfer from holo IscU to apo Fd in the presence of chaperone DnaK demonstrate an inhibitory effect on the rate of Fe-S cluster transfer from IscU. Iron 148-152 iron-sulfur cluster assembly enzyme Homo sapiens 175-179 15766257-6 2005 Rather, DnaK has a modest influence on the stability of the IscU-bound Fe-S cluster that may reflect a more important role in promoting cluster assembly. Iron 71-75 iron-sulfur cluster assembly enzyme Homo sapiens 60-64 15247288-0 2004 IscA mediates iron delivery for assembly of iron-sulfur clusters in IscU under the limited accessible free iron conditions. Iron 44-48 iron-sulfur cluster assembly enzyme Homo sapiens 68-72 15247288-0 2004 IscA mediates iron delivery for assembly of iron-sulfur clusters in IscU under the limited accessible free iron conditions. Iron 44-48 iron-sulfur cluster assembly enzyme Homo sapiens 68-72 15247288-1 2004 Increasing evidence suggests that IscS, a cysteine desulfurase, provides sulfur for assembly of transient iron-sulfur clusters in IscU. Iron 106-110 iron-sulfur cluster assembly enzyme Homo sapiens 130-134 15247288-4 2004 Here we find that Escherichia coli IscU fails to assemble iron-sulfur clusters when the accessible "free" iron in solution is limited by an iron chelator sodium citrate. Iron 58-62 iron-sulfur cluster assembly enzyme Homo sapiens 35-39 15247288-4 2004 Here we find that Escherichia coli IscU fails to assemble iron-sulfur clusters when the accessible "free" iron in solution is limited by an iron chelator sodium citrate. Iron 106-110 iron-sulfur cluster assembly enzyme Homo sapiens 35-39 15247288-5 2004 Remarkably, IscA, an iron-sulfur cluster assembly protein with an iron association constant of 3.0 x 10(19) m(-1), is able to overcome the iron limitation due to sodium citrate and deliver iron for the IscS-mediated iron-sulfur cluster assembly in IscU. Iron 21-25 iron-sulfur cluster assembly enzyme Homo sapiens 248-252 15247288-5 2004 Remarkably, IscA, an iron-sulfur cluster assembly protein with an iron association constant of 3.0 x 10(19) m(-1), is able to overcome the iron limitation due to sodium citrate and deliver iron for the IscS-mediated iron-sulfur cluster assembly in IscU. Iron 66-70 iron-sulfur cluster assembly enzyme Homo sapiens 248-252 15247288-5 2004 Remarkably, IscA, an iron-sulfur cluster assembly protein with an iron association constant of 3.0 x 10(19) m(-1), is able to overcome the iron limitation due to sodium citrate and deliver iron for the IscS-mediated iron-sulfur cluster assembly in IscU. Iron 66-70 iron-sulfur cluster assembly enzyme Homo sapiens 248-252 15247288-5 2004 Remarkably, IscA, an iron-sulfur cluster assembly protein with an iron association constant of 3.0 x 10(19) m(-1), is able to overcome the iron limitation due to sodium citrate and deliver iron for the IscS-mediated iron-sulfur cluster assembly in IscU. Iron 66-70 iron-sulfur cluster assembly enzyme Homo sapiens 248-252 15247288-5 2004 Remarkably, IscA, an iron-sulfur cluster assembly protein with an iron association constant of 3.0 x 10(19) m(-1), is able to overcome the iron limitation due to sodium citrate and deliver iron for the IscS-mediated iron-sulfur cluster assembly in IscU. Iron 66-70 iron-sulfur cluster assembly enzyme Homo sapiens 248-252 15247288-8 2004 The results suggest that IscA is capable of recruiting intracellular iron and providing iron for the iron-sulfur cluster assembly in IscU in cells in which the accessible "free" iron content is probably restricted. Iron 88-92 iron-sulfur cluster assembly enzyme Homo sapiens 133-137 15247288-8 2004 The results suggest that IscA is capable of recruiting intracellular iron and providing iron for the iron-sulfur cluster assembly in IscU in cells in which the accessible "free" iron content is probably restricted. Iron 88-92 iron-sulfur cluster assembly enzyme Homo sapiens 133-137 15247288-8 2004 The results suggest that IscA is capable of recruiting intracellular iron and providing iron for the iron-sulfur cluster assembly in IscU in cells in which the accessible "free" iron content is probably restricted. Iron 88-92 iron-sulfur cluster assembly enzyme Homo sapiens 133-137 12886008-3 2003 In particular, genetic and biochemical studies suggest that IscU, Nfu, and IscA function as scaffold proteins for assembly and delivery of rudimentary Fe-S clusters to target proteins. Iron 151-155 iron-sulfur cluster assembly enzyme Homo sapiens 60-64 15143178-10 2004 Further, the bacterial homologue IscU and the human Isu proteins (partially) complemented the defects of yeast Isu protein-depleted cells in growth rate, Fe/S protein biogenesis, and iron homeostasis, yet only after targeting to mitochondria. Iron 183-187 iron-sulfur cluster assembly enzyme Homo sapiens 33-37 14993221-0 2004 Iron-sulfur cluster assembly: NifU-directed activation of the nitrogenase Fe protein. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 30-34 14720122-2 2004 At least six proteins (IscS, IscU, IscA, HscB, HscA and ferredoxin) have been identified as being essential for the biogenesis of iron-sulphur proteins in bacteria. Iron 130-134 iron-sulfur cluster assembly enzyme Homo sapiens 29-33 14720122-3 2004 It has been shown that IscS is a cysteine desulphurase that provides sulphur for iron-sulphur clusters, and that IscU is a scaffold for the IscS-mediated assembly of iron-sulphur clusters. Iron 166-170 iron-sulfur cluster assembly enzyme Homo sapiens 113-117 14720122-5 2004 Here we show that IscA is an iron binding protein with an apparent iron association constant of 3.0x10(19) M(-1), and that iron-loaded IscA can provide iron for the assembly of transient iron-sulphur clusters in IscU in the presence of IscS and L-cysteine in vitro. Iron 29-33 iron-sulfur cluster assembly enzyme Homo sapiens 212-216 14720122-5 2004 Here we show that IscA is an iron binding protein with an apparent iron association constant of 3.0x10(19) M(-1), and that iron-loaded IscA can provide iron for the assembly of transient iron-sulphur clusters in IscU in the presence of IscS and L-cysteine in vitro. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 212-216 14720122-5 2004 Here we show that IscA is an iron binding protein with an apparent iron association constant of 3.0x10(19) M(-1), and that iron-loaded IscA can provide iron for the assembly of transient iron-sulphur clusters in IscU in the presence of IscS and L-cysteine in vitro. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 212-216 14720122-5 2004 Here we show that IscA is an iron binding protein with an apparent iron association constant of 3.0x10(19) M(-1), and that iron-loaded IscA can provide iron for the assembly of transient iron-sulphur clusters in IscU in the presence of IscS and L-cysteine in vitro. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 212-216 14720122-5 2004 Here we show that IscA is an iron binding protein with an apparent iron association constant of 3.0x10(19) M(-1), and that iron-loaded IscA can provide iron for the assembly of transient iron-sulphur clusters in IscU in the presence of IscS and L-cysteine in vitro. Iron 67-71 iron-sulfur cluster assembly enzyme Homo sapiens 212-216 12886008-9 2003 These results, together with previous reports of subcellular distributions of isoforms of human IscS and IscU in mitochondria, cytosol, and nucleus suggest that the Fe-S cluster assembly machineries are compartmentalized in higher eukaryotes. Iron 165-169 iron-sulfur cluster assembly enzyme Homo sapiens 105-109 12756240-7 2003 These findings are consistent with the role in Fe/S biogenesis previously proposed for the bacterial Hsp70 Hsc66 and J-protein Hsc20 that interact with the bacterial Isu homologue IscU. Iron 47-49 iron-sulfur cluster assembly enzyme Homo sapiens 180-184 12137512-0 2002 Iron-sulfur cluster biosynthesis: characterization of iron nucleation sites for assembly of the [2Fe-2S]2+ cluster core in IscU proteins. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 123-127 12137512-0 2002 Iron-sulfur cluster biosynthesis: characterization of iron nucleation sites for assembly of the [2Fe-2S]2+ cluster core in IscU proteins. Iron 54-58 iron-sulfur cluster assembly enzyme Homo sapiens 123-127 12137512-2 2002 The intermediate [2Fe-2S] IscU-bound cluster is formed by delivery of iron and sulfur to the apo-IscU, with the latter delivered through an IscS-mediated reaction. Iron 70-74 iron-sulfur cluster assembly enzyme Homo sapiens 26-30 12137512-2 2002 The intermediate [2Fe-2S] IscU-bound cluster is formed by delivery of iron and sulfur to the apo-IscU, with the latter delivered through an IscS-mediated reaction. Iron 70-74 iron-sulfur cluster assembly enzyme Homo sapiens 97-101 12137512-4 2002 In this report we characterize iron-binding sites on IscU that appear to nucleate [2Fe-2S] cluster assembly. Iron 31-35 iron-sulfur cluster assembly enzyme Homo sapiens 53-57 12137512-5 2002 This iron-bound form of IscU is shown to be viable for subsequent IscS-mediated assembly of holo-IscU. Iron 5-9 iron-sulfur cluster assembly enzyme Homo sapiens 24-28 12137512-5 2002 This iron-bound form of IscU is shown to be viable for subsequent IscS-mediated assembly of holo-IscU. Iron 5-9 iron-sulfur cluster assembly enzyme Homo sapiens 97-101 12137512-7 2002 The latter observation reflects the low binding affinity of persulfido IscU for iron ion. Iron 80-84 iron-sulfur cluster assembly enzyme Homo sapiens 71-75 11060020-3 2000 Here we report cloning of the human homologs to IscU and NifU, iron-binding proteins that play a critical role in Fe-S cluster assembly in bacteria. Iron 63-67 iron-sulfur cluster assembly enzyme Homo sapiens 48-52 11686732-0 2001 Sulfur transfer from IscS to IscU: the first step in iron-sulfur cluster biosynthesis. Iron 53-57 iron-sulfur cluster assembly enzyme Homo sapiens 29-33 11123951-1 2000 The Fe-S cluster formation proteins NifU and NifS are essential for viability in the ulcer causing human pathogen Helicobacter pylori. Iron 4-8 iron-sulfur cluster assembly enzyme Homo sapiens 36-40 11123951-6 2000 Purified NifU is a red protein that contains approximately 1.5 atoms of iron per monomer. Iron 72-76 iron-sulfur cluster assembly enzyme Homo sapiens 9-13 11123951-8 2000 The primary structure of NifU also contains the three conserved cysteine residues which are involved in providing the scaffold for the assembly of a transient Fe-S cluster for insertion into apoprotein. Iron 159-163 iron-sulfur cluster assembly enzyme Homo sapiens 25-29 11123951-11 2000 The results here indicate that the NifU type of Fe-S cluster formation proteins is not specific for maturation of the nitrogenase proteins and, as H. pylori lacks other Fe-S cluster assembly proteins, that the H. pylori NifS and NifU are responsible for the assembly of many (non-nitrogenase) Fe-S clusters. Iron 48-52 iron-sulfur cluster assembly enzyme Homo sapiens 35-39 11123951-11 2000 The results here indicate that the NifU type of Fe-S cluster formation proteins is not specific for maturation of the nitrogenase proteins and, as H. pylori lacks other Fe-S cluster assembly proteins, that the H. pylori NifS and NifU are responsible for the assembly of many (non-nitrogenase) Fe-S clusters. Iron 48-52 iron-sulfur cluster assembly enzyme Homo sapiens 229-233 11123951-11 2000 The results here indicate that the NifU type of Fe-S cluster formation proteins is not specific for maturation of the nitrogenase proteins and, as H. pylori lacks other Fe-S cluster assembly proteins, that the H. pylori NifS and NifU are responsible for the assembly of many (non-nitrogenase) Fe-S clusters. Iron 169-173 iron-sulfur cluster assembly enzyme Homo sapiens 35-39 11060020-3 2000 Here we report cloning of the human homologs to IscU and NifU, iron-binding proteins that play a critical role in Fe-S cluster assembly in bacteria. Iron 63-67 iron-sulfur cluster assembly enzyme Homo sapiens 57-61 11060020-3 2000 Here we report cloning of the human homologs to IscU and NifU, iron-binding proteins that play a critical role in Fe-S cluster assembly in bacteria. Iron 114-116 iron-sulfur cluster assembly enzyme Homo sapiens 48-52 11060020-3 2000 Here we report cloning of the human homologs to IscU and NifU, iron-binding proteins that play a critical role in Fe-S cluster assembly in bacteria. Iron 114-116 iron-sulfur cluster assembly enzyme Homo sapiens 57-61 11060020-5 2000 Biochemical analyses demonstrate that IscU proteins specifically associate with IscS, a cysteine desulfurase that is proposed to sequester inorganic sulfur for Fe-S cluster assembly. Iron 160-162 iron-sulfur cluster assembly enzyme Homo sapiens 38-42 11060020-6 2000 Protein complexes containing IscU and IscS can be found in the mitochondria as well as in the cytosol, implying that Fe-S cluster assembly takes place in multiple subcellular compartments in mammalian cells. Iron 117-121 iron-sulfur cluster assembly enzyme Homo sapiens 29-33 32776106-2 2020 In this work, using genetic and biochemical approaches, we identified the initial scaffold protein, mitochondrial ISCU (ISCU2) and the secondary carrier, ISCA1, as the direct donors of Fe-S clusters to mitochondrial NFU1, which appears to dimerize and reductively mediate formation of a bridging [4Fe-4S] cluster, aided by ferredoxin 2 (FDX2). Iron 185-187 iron-sulfur cluster assembly enzyme Homo sapiens 114-118 10891064-0 2000 IscU as a scaffold for iron-sulfur cluster biosynthesis: sequential assembly of [2Fe-2S] and [4Fe-4S] clusters in IscU. Iron 23-27 iron-sulfur cluster assembly enzyme Homo sapiens 0-4 10891064-1 2000 Iron-sulfur cluster biosynthesis in both prokaryotic and eukaryotic cells is known to be mediated by two highly conserved proteins, termed IscS and IscU in prokaryotes. Iron 0-4 iron-sulfur cluster assembly enzyme Homo sapiens 148-152 10891064-3 2000 In this work, the time course of IscS-mediated Fe-S cluster assembly in IscU was monitored via anaerobic anion exchange chromatography. Iron 47-51 iron-sulfur cluster assembly enzyme Homo sapiens 72-76 10891064-8 2000 In addition, the resonance Raman spectrum of the [4Fe-4S](2+) cluster in IscU is best interpreted in terms of noncysteinyl ligation at a unique Fe site. Iron 51-53 iron-sulfur cluster assembly enzyme Homo sapiens 73-77 26560363-0 2015 Regulation of iron homeostasis by the p53-ISCU pathway. Iron 14-18 iron-sulfur cluster assembly enzyme Homo sapiens 42-46 26560363-2 2015 Here, we report that p53 regulates iron metabolism through the transcriptional regulation of ISCU (iron-sulfur cluster assembly enzyme), which encodes a scaffold protein that plays a critical role in Fe-S cluster biogenesis. Iron 35-39 iron-sulfur cluster assembly enzyme Homo sapiens 93-97 26560363-2 2015 Here, we report that p53 regulates iron metabolism through the transcriptional regulation of ISCU (iron-sulfur cluster assembly enzyme), which encodes a scaffold protein that plays a critical role in Fe-S cluster biogenesis. Iron 99-103 iron-sulfur cluster assembly enzyme Homo sapiens 93-97 26560363-2 2015 Here, we report that p53 regulates iron metabolism through the transcriptional regulation of ISCU (iron-sulfur cluster assembly enzyme), which encodes a scaffold protein that plays a critical role in Fe-S cluster biogenesis. Iron 200-202 iron-sulfur cluster assembly enzyme Homo sapiens 93-97 26560363-4 2015 Knockdown of ISCU enhanced the binding of iron regulatory protein 1 (IRP1), a cytosolic Fe-S protein, to an iron-responsive element in the 5" UTR of ferritin heavy polypeptide 1 (FTH1) mRNA and subsequently reduced the translation of FTH1, a major iron storage protein. Iron 42-46 iron-sulfur cluster assembly enzyme Homo sapiens 13-17 26560363-4 2015 Knockdown of ISCU enhanced the binding of iron regulatory protein 1 (IRP1), a cytosolic Fe-S protein, to an iron-responsive element in the 5" UTR of ferritin heavy polypeptide 1 (FTH1) mRNA and subsequently reduced the translation of FTH1, a major iron storage protein. Iron 108-112 iron-sulfur cluster assembly enzyme Homo sapiens 13-17 26560363-9 2015 Our finding revealed a novel role of the p53-ISCU pathway in the maintenance of iron homeostasis in hepatocellular carcinogenesis. Iron 80-84 iron-sulfur cluster assembly enzyme Homo sapiens 45-49 34199378-6 2021 These details support a complex dynamic interaction between the FXN and ISCU proteins when both are part of the NIAUF complex and this provides additional insight into the coordinated mechanism of Fe-S cluster assembly. Iron 197-199 iron-sulfur cluster assembly enzyme Homo sapiens 72-76 35079622-0 2022 FDX2 and ISCU Gene Variations Lead to Rhabdomyolysis With Distinct Severity and Iron Regulation. Iron 80-84 iron-sulfur cluster assembly enzyme Homo sapiens 9-13 35079622-9 2022 Discussion: We conclude that FDX2 and ISCU variants result in a similar muscle phenotype, that differ in severity and skeletal muscle iron accumulation. Iron 134-138 iron-sulfur cluster assembly enzyme Homo sapiens 38-42 32776106-2 2020 In this work, using genetic and biochemical approaches, we identified the initial scaffold protein, mitochondrial ISCU (ISCU2) and the secondary carrier, ISCA1, as the direct donors of Fe-S clusters to mitochondrial NFU1, which appears to dimerize and reductively mediate formation of a bridging [4Fe-4S] cluster, aided by ferredoxin 2 (FDX2). Iron 185-187 iron-sulfur cluster assembly enzyme Homo sapiens 120-125 32202031-6 2020 These include NifS and NifU, found primarily in aerobic species, suggesting that these genes are necessary for accommodating the high demand for Fe-S clusters during aerobic nitrogen fixation. Iron 145-149 iron-sulfur cluster assembly enzyme Homo sapiens 23-27